Pulse Doses Of Methylprednisolone Research Articles

The impact of different corticosteroids in hospitalised patients with severe COVID-19 pneumonia: a retrospective observational study

Background: The COVID-19 pandemic created havoc on health care systems and economies worldwide. Corticosteroids have been shown to be effective in the management of COVID-19. Methods: A retrospective observational study was conducted on 311 patients meeting the inclusion criteria at the Augustus Long Hospital, Trinidad and Tobago between July-December 2021. Data was classified into three treatment groups: low dose dexamethasone, high dose dexamethasone and methylprednisolone pulse therapy. The primary outcomes were the 28-day mortality and need for invasive mechanical ventilation and the secondary outcome was the length of hospital stay. The patient cohort was also classified into simple oxygen and high flow oxygen groups and a subgroup analysis was also performed. Results: There were no significant statistical differences between age, gender, vaccination status and co-morbidities between the treatment groups. However, there were significant differences in the ethnic ratios amongst the high dose dexamethasone and methylprednisolone treatment groups (East Indians to Africans; 61.7%vs. 37%; 72.9% vs.25.4% respectively; p=0.02). In patients receiving simple oxygen on admission, those who were treated with low dose dexamethasone when compared to high dose dexamethasone and methylprednisolone, had the lowest 28-day mortality (15.3% vs. 40.9% vs. 40% respectively; p< 0.001), lowest need for IMV (0.7% vs. 9.1% vs. 40% respectively; p< 0.001) and the shortest length of hospital stay (median [IQR] days; 7[12-5], 11[22-7], 16[27-14] respectively; p< 0.001). In patients receiving high flow oxygen, those treated with methylprednisolone had the lowest 28-day mortality (45.8%), when compared to the high dose dexamethasone (59.5%) and low dose dexamethasone (81%) groups; however, these results were not statistically significant (p = 0.053). Conclusion: In our study, patients receiving simple oxygen had better clinical outcomes when treated with low dose dexamethasone compared to higher dose corticosteroids. Methylprednisolone pulse therapy may be beneficial in patients requiring high flow oxygen; but further studies are needed.

Steroid-responsive encephalopathy in autoimmune thyroiditis in a 2-year-old child after COVID-19 infection: A case report

In pediatric patients, neurological manifestation of COVID-19 infection has been reported in acute and postinfectious stages. Hashimoto encephalopathy (HE) is the most common cause of hypothyroidism in children with prevalence being 1.2%. More than 200 cases of HE have been reported in adults. Only 60 cases have been reported in children. We present a case of a 2-year-old girl with status epilepticus and respiratory failure needing prolonged ventilation. Her COVID-19 antibody and antithyroid antibodies (anti-thyroid peroxidase and anti-thyroglobulin) were positive. Her thyroid profile was abnormal. Her clinical state improved after receiving an intravenous immunoglobulin and pulse dose of methylprednisolone. This suggests that complete recovery for such patients can be achieved with early detection and immunomodulatory treatment.

Pre-hospital pulse glucocorticoid therapy in patients with ST-segment elevation myocardial infarction transferred for primary percutaneous coronary intervention: a randomized controlled trial (PULSE-MI)

BackgroundInflammation in ST-segment elevation myocardial infarction (STEMI) is an important contributor to both acute myocardial ischemia and reperfusion injury after primary percutaneous coronary intervention (PCI). Methylprednisolone is a glucocorticoid with potent anti-inflammatory properties with an acute effect and is used as an effective and safe treatment of a wide range of acute diseases. The trial aims to investigate the cardioprotective effects of pulse-dose methylprednisolone administered in the pre-hospital setting in patients with STEMI transferred for primary PCI.MethodsThis trial is a randomized, blinded, placebo-controlled prospective clinical phase II trial. Inclusion will continue until 378 patients with STEMI have been evaluated for the primary endpoint. Patients will be randomized 1:1 to a bolus of 250 mg methylprednisolone intravenous or matching placebo over a period of 5 min in the pre-hospital setting. All patients with STEMI transferred for primary PCI at Rigshospitalet, Copenhagen University Hospital, Denmark, will be screened for eligibility. The main eligibility criteria are age ≥ 18 years, acute onset of chest pain with < 12 h duration, STEMI on electrocardiogram, no known allergy to glucocorticoids or no previous coronary artery bypass grafting, previous acute myocardial infarction in assumed culprit, or a history with previous maniac/psychotic episodes. Primary outcome is final infarct size measured by late gadolinium enhancement on cardiac magnetic resonance (CMR) 3 months after STEMI. Secondary outcomes comprise key CMR efficacy parameters, clinical endpoints at 3 months, the peak of cardiac biomarkers, and safety.DiscussionWe hypothesize that pulse-dose methylprednisolone administrated in the pre-hospital setting decreases inflammation and thus reduces final infarct size in patients with STEMI treated with primary PCI.Trial registrationEU-CT number: 2022–500762-10–00; Submitted May 5, 2022. ClinicalTrials.gov Identifier: NCT05462730; Submitted July 7, 2022, first posted July 18, 2022.

SAT624 Neurosarcoidosis Presenting With Panhypopituitarism

Abstract Disclosure: E.M. Niedzialkowska: None. T. Blazin: None. K. Strubchevska: None. M. Kozyk: None. N. Saini: None. Introduction: Neurosarcoidosis is estimated to occur in approximately 5-15% patients with sarcoidosis. Pituitary involvement may be present in up to 5% of those cases and can manifest as hypothalamus, adenohypophysis dysfunction or arginine vasopressin deficiency (AVP-D) in isolated fashion or variedly combined. Clinical case: 32 year old male presented with chief complaint of worsening bilateral lower extremity weakness, urinary incontinence, polyuria and polydipsia. 9 months prior to admission the patient was diagnosed with neurosarcoidosis. At that time brain MRI showed diffuse leptomeningeal enhancement, chest CT showed hilar lymphadenopathy, lumbar puncture with CSF analysis showed protein 227 mg/dl (N&amp;lt;38), ACE 6 U/l (N &amp;lt;2.5), WBC 10 (N&amp;lt;5) with 88% lymphocytes. Hilar lymph node biopsy was consistent with non-caseating granuloma. The patient was diagnosed with neurosarcoidosis and started on prednisone and methotrexate. The patient was referred to our institution for further evaluation. On examination gait ataxia and gynecomastia were noted. Laboratory results showed urine specific gravity of 1.001, LH 0.4 mIU/ml (N 0.6-1.2), FSH 1.1 mIU/ml (N 1-12), ACTH &amp;lt;5 pg/ml (N 6-46), cortisol 2.1 ug/dl (N 2.9-19.4), prolactin 10.5 ng/ml (N 2-18), total testosterone 4 ng/dl (N 240-1080), free testosterone 0.1 ng/dl (N 4.8-19), TSH 0.58 uIU/ml (N 0.4-4.5), free ft3 1.5 pg/ml (N 1.7-3.7), free ft4 0.6 ng/dl (N 0.7-1.5), IGF-1 52 ng/ml (N 71-234), Copeptin proAVP of &amp;lt;2.8 pmol/l( N&amp;gt;13.2), urine osmolality of 72 mOsmol/kg, blood osmolality of 297 mOsmol/kg (N &amp;lt;295), vit D 25-OH 9 ng/ml (N 30-100), vit D 1,25-OH 57.3 pg/ml (N 19.9-79.3). MRI of the spine showed extensive nodular enhancement of the leptomeninges of the cervical, thoracic spine and lumbar cauda equina nerve roots. Brain MRI was positive for numerous small nodular enhancement involving leptomeninges and pituitary stalk. The patient was started on desmopressin with significant improvement of symptoms and increase in urine osmolality to 709 mOsmol/kg. Due to risk of hypercalcemia and normal vit D 1,25-OH vitamin D supplementation was not initiated. For neurosarcoidosis the patient was started on pulse dose methylprednisolone and infliximab infusion. Upon discharge he was transitioned to oral hydrocortisone and started on levothyroxine and testosterone replacement therapy. Conclusion: We present a case of pituitary neurosarcoidosis presenting with AVP-D, secondary hypothyroidism, secondary adrenal insufficiency, hypogonadism and growth hormone deficiency. Panhypopituitarism with complete anterior and posterior pituitary dysfunction is extremely rare and might be found in up to 13% cases of pituitary sarcoidosis. The diagnosis should be considered in patients with sarcoidosis and endocrine dysfunctions. Presentation: Saturday, June 17, 2023

Predicting systemic lupus erithematosus disease activity index 2000 (Sledai 2k) score after pulse dose metyl prednisolone in severe systemic lupus erithematosus

Background: Systemic lupus erythematosus (SLE) is a chronic multisystem rheumatic disease characterized by developing autoantibodies against nucleus antigen. It has a broad range of clinical symptoms and the potential to affect nearly all organs and tissues. Pulse dosage methylprednisolone (MEP) is the immunosuppression modality for life-threatening or organ-threatening SLE. However, it is challenging to estimate the MEP response rate. We combine clinical symptoms, routine laboratory examinations, and more specific protein examinations such as soluble B-cell activating factor (sBAFF), B-cell activating factor receptor (BAFF-R), and Interferon gamma-induced protein 10 (IP-10) to develop a formula that can predict the SLE Disease Activity Index 2K (SLEDAI 2K) score following a pulse dosage of methylprednisolone. Methods: In a prospective cohort study, patients with severe SLE with a SLEDAI 2K score of 12 or Lupus nephritis class III or IV according to WHO criteria were given methylprednisolone 500 mg/day for three consecutive days. Enzyme-linked immunosorbent assay (ELISA) tested blood samples for soluble (s) BAFF, IP 10, and flow cytometry for BAFF-R, CD-19. The SLEDAI 2K score was reevaluated after a pulse dose of methylprednisolone was administered. All statistical analyses were conducted using the Rstudio program. Results: Overall, 80 patients were included. Multivariate multiple regression analysis revealed that urine protein creatinine ratio (UPCR) (x1), CD19 percentage (x2), serum BAFF (x3), vasculitis (x4), and rash (x5) taken before MEP pulse were predictors for SLEDAI 2k score after pulse dose methylprednisolone in severe SLE with the formula 13.41+ (0.0008542 * x1) + -0.1829338 * x2) + (0.0008776 * x3) + (7.1801728 * x4) + (7.5429676 * x6). Conclusions: Formula to predict SLEDAI 2k score after MEP pulse was 13.41+ (0.0008542 * x1) + -0.1829338 * x2) + (0.0008776 * x3) + (7.1801728 * x4) + (7.5429676 * x6). Further validation is needed to be used in clinical practice.

Pulse Dose Methylprednisolone Therapy in a Cohort of Very Severe COVID-19 Patients in a Resource-limited Setting in Myanmar: A Case Series of 13 Patients

Aims: Coronavirus disease 2019 (COVID-19) has very high mortality in severe forms of disease, where immunopathology plays an important role. Use of immunomodulating therapies including 6 to 12 mg of dexamethasone is well established. Higher doses of corticosteroids were used with reported success in some settings. This study aims to explore the role of pulse dose methylprednisolone therapy in very severe COVID-19 patients in preventing the need for ICU care and death in resource-limited setting.&#x0D; Study Design: Retrospective case series study.&#x0D; Place and Duration of Study: Oak-ta-chat-thal-ta-pwint COVID-19 treatment center in Yangon, Myanmar between September 2021 to December 2021.&#x0D; Methodology: This study included 13 confirmed COVID-19 patients with severe to critical illness, who were treated with pulse dose methylprednisolone therapy. We reviewed the patients’ demographics, comorbidities, and disease severity before starting pulse dose methylprednisolone therapy and changes in oxygen requirement, chest X-ray scores, inflammatory markers, development of significant clinical events, and 28 days mortality after therapy.&#x0D; Results: Before pulse dose methylprednisolone therapy, all 13 patients had very severe disease (mean SPO2/FiO2 = 173 mmHg, mean SPO2 = 88.54%, mean CRP = 115 mg/L, mean ferritin = 1,295.5 ng/mL and mean Brixia Score = 6.54). They received 3-7 days (mean = 5.5 days) of pulse dose methylprednisolone. Ten patients (76%) survived in a setting with limited ICU care. High ferritin was a significant predictor of mortality. Improvement in oxygen requirement was noticeable after 1-11 days (mean = 5.6 days). Hyperglycemia was common and confirmed bacterial infection was found in 3 patients, but all patients received empirical antibiotics therapy.&#x0D; Conclusion: Pulse-dose methylprednisolone therapy may be an effective salvage therapy in a carefully selected subset of very severe COVID-19 patients. It might be a feasible alternative to other more expensive immunomodulating agents and organ support treatments in a resource-limited setting.

A Rare Presentation of Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis with Neuropsychiatric Symptoms: A Case Report

Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune disease that appears as a fulminant, subacute or chronic course of altered mental status, often accompanied by seizures and myoclonus. This case report demonstrates that SREAT can be present with solitary neuropsychiatric signs long before seizures and myoclonus. Methods: A 42-year-old female presented with abrupt-onset whole-body myoclonic jerks. She had been suffering from depression for 15 years before being diagnosed with hypothyroidism. She was conscious, oriented and alert. At rest, all four limbs had multifocal mild-to-moderate myoclonus, which was significantly aggravated by muscle activation; her Hamilton Depression Rating Scale score was 21. Results: Antithyroglobulin and antithyroid peroxidase autoantibodies were both above 2,000 IU/mL. The thyroid-stimulating hormone level was 5.65 mIU/mL, free triiodothyronine level was 3.36 pg/mL and free thyroxine level was 1.02 ng/dL. Vasculitis profile and the serum test for autoimmune encephalitis panel were negative. Brain neuroimaging was normal. Pulse dose of methylprednisolone followed by oral steroids resulted in significant clinical improvement. Conclusion: SREAT can present with chronic neuropsychiatric symptoms with abrupt exacerbation of seizures and myoclonus. This case study emphasizes the importance of screening individuals with depression and thyroid problems for serum antithyroid antibody levels. In most cases, steroid treatment yields positive results.

Anti-N-Methyl-D-Aspartate-Receptor Encephalitis Following Herpes Simplex Virus Encephalitis – Presenting as a Pediatric Patient with Abnormal Movements and Psychiatric Manifestation

Herpes simplex virus (HSV) encephalitis remains one of the more severe forms of childhood encephalitis. The clinical course of HSV encephalitis is usually monophasic but occasionally complicated by a clinical relapse, causing biphasic illness. Sometimes relapse may be due to secondary immune mechanisms. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an example of immune-mediated relapse following herpes encephalitis. This is still underrecognized, with probable grave consequences if not treated early. Our index case was an 8-year-old male child with severe generalized choreoathetosis and behavioral disturbances, presented 4 weeks after documented HSV encephalitis. Cerebrospinal fluid (CSF) was positive for anti-NMDA receptor antibodies. The diagnosis was confirmed and treated with intravenous (IV) immunoglobulins (2 g/kg) and IV pulse-dose methylprednisolone (30 mg/kg) for 5 days. Other supportive management included tablet revocon 25 mg (tetrabenazine) ¼ tablet BD, clonazepam 0.25 mg one tablet BD, and syrup sizodon (risperidone) 0.5 mg BD. The patient is on regular follow-up and is recovering well but slowly. Patients with relapsing symptoms following HSV encephalitis or prolonged atypical symptoms, with repeat CSF-negative polymerase chain reaction for HSV should routinely be tested for NMDA receptor immunoglobulin G antibodies in CSF and/or serum. It is important to be aware of this differential diagnosis because patients respond well to immunotherapy.

S3211 Weight Loss, Ascites, and Diarrhea as the Presenting Symptoms for Pseudo-Pseudo Meigs Syndrome

Introduction: Pseudo-pseudo Meigs syndrome (PPMS) is a rare presentation of systemic lupus erythematous (SLE) characterized by ascites, pleural effusion, and elevated CA-125 level. Case Description/Methods: A 30-year-old woman with SLE and history of small bowel obstruction presented with diarrhea, weight loss, anasarca, and alopecia. She was initially tachycardic and hypertensive. Her exam revealed profound anasarca with ascites and bilateral pleural effusions. Labs were notable for Cr=5.65 mg/dL (baseline 0.7 mg/dL), spot protein/creatinine=3.9, ferritin=554.6 ng/ml, ESR=145 mm/Hr, CRP=6 mm/Hr, C3=49 mg/dL, C4=19 mg/dL, unremarkable FSH and LH, +ANA, dsDNA 356 U/ml, and +pANCA/MPO. Outpatient workup showed elevated CA-125 to 260.8 U/mL but ovarian malignancy was ruled out by MRI and CT abdomen/pelvis. Transvaginal ultrasound was normal. Inflammatory bowel disease was ruled out by EGD and colonoscopy during a previous hospitalization. Renal ultrasound showed bilateral hydronephrosis. The patient underwent a paracentesis (SAAG 1.3), and thoracentesis that showed total serum protein 4.2 g/dl, pleural fluid LDH 67 U/L, no serum LDH or pleural protein available. Kidney biopsy demonstrated class IV / V lupus nephritis. She was treated with a three-day course of pulse dose methylprednisolone followed by prednisone, hydroxychloroquine, and furosemide infusion. Her symptoms mostly resolved and CA-125 trended down to 27 U/mL. Discussion: PPMS is a rare condition with few cases reported in the literature. This case illustrates the challenges of evaluating abdominal symptoms and ascites in patients with SLE, and the importance of a kidney biopsy in the evaluation of nephrotic range proteinuria. Due to infrequency and concern for malignancy due to elevated CA-125 level, patients with this syndrome undergo extensive workup. CA-125 has been shown to be elevated due to inflammatory markers enhancing its expression in peritoneal mesothelial cells. Prior publications have shown the correlation of CA-125 with extent of serositis in PPMS. Additionally, our patient has many other clinical features reported in PPMS patients including elevated ferritin, alopecia, and positive pANCA. CT scan of abdomen and pelvis is essential in the workup of this syndrome to rule out ovarian malignancy. Moreover, imaging may exclude mesenteric vasculitis, an uncommon feature of SLE which can be seen as targetoid lesions of the bowel on CT scan. Steroids are the treatment for PPMS.

Multisystem inflammatory syndrome in children in Coastal Andhra, India

Background: Multisystem inflammatory syndrome in children (MIS-C) associated with COVID is a recently emerged disease and there is limited Indian data. We reported the presentation and clinical course of these children.Methods: All children who had symptoms and signs of MIS-C according to WHO criteria and those positive for SARS-CoV-2, were considered for the study. Clinical features, lab parameters, treatment details and outcome were documented.Results: 104 children, who had MIS-C during the study period. Out of them, 52 (50%) required respiratory support. Among those who required respiratory support 13 (12%) children required invasive ventilation, 11 (10.5%) children could be managed with noninvasive ventilation, whereas 28 (27%) children required supplemental oxygen. 18 (17%) children had signs of myocarditis and left ventricular dysfunction. 12 (11.5%) children had pulmonary edema and Coronary artery dilatation was present in 4(4%) cases. Steroids were used as main stay of therapy. Low dose intravenous methylprednisolone was administered to72(69%); pulse dose methylprednisolone was administered to 12 (12%) children. Intravenous immunoglobulins (IVIG) administered to 20 (19%) children. Among the study group, 103 children survived and one child expired (0.96%).Conclusions: Optimal use of immunomodulators and frequent hemodynamic assessment with functional ECHO to optimize the inotropes, can improve the outcomes among those with myocardial dysfunction.

Evaluation of personalized methylprednisolone therapy in critically ill COVID-19 patients: an observational comparative study using real-life data.

Methylprednisolone is commonly used to attenuate the cytokine storm and prevent mortality in COVID-19 pneumonia. However, the optimal methylprednisolone dose and duration are unclear. Additional data are required on the effectiveness of methylprednisolone in reducing mortality in COVID-19. This real-life retrospective study aimed to analyze the data of a COVID-19 dedicated ICU and compare the mortality rates of standard care, low-dose, and pulse-dose methylprednisolone in patients requiring mechanical ventilatory support. Methylprednisolone's indication, dose, and duration were determined according to the severity of COVID-19 pneumonia based on the patient's demographic parameters, comorbidities, laboratory data, radiology, and arterial blood gas analysis results. 867 patients were grouped as: no methylprednisolone (standard care), low-dose (0.5-1 mg/kg/day) methylprednisolone or pulse-dose (250-1,000 mg/day) methylprednisolone. The overall mortality rate was 63.78%. Adjusting the dose of methylprednisolone according to the severity of the disease resulted in statistically similar mortality rates despite the increase in disease severity. Mortality was 62.71% in standard treatment, 65.76% in low-dose, and 62.10% in pulse-dose methylprednisolone groups (p = 0.633). Invasive mechanical ventilation at admission was associated with increased mortality (HR: 1.826 [95% CI: 1.542-2.161]; p < 0.001). Hematologic disorders and malignancies, arterial blood pH and HCO3, neutrophil count, and NLR at admission were also associated with mortality. Personalizing the dose and duration of methylprednisolone according to the patient's disease severity assessed with demographic, clinical, and laboratory results may benefit mortality in severe COVID-19 patients receiving ventilatory support in the ICU. Hematologic disorders and malignancies, arterial blood pH and HCO3, neutrophil count, and NLR at admission were associated with mortality in our patient cohort.

A young adult with multisystem inflammatory syndrome (MIS-A) following COVID-19 infection: A case report

Recent reports have described a secondary Multi system Inflammatory Syndrome in adult (MIS-A)and its temporal association with COVID-19 infection. Here, we report the case of a 25-year-old male who presented with shock and multi organ failure. Three-week prior to present admission he developed an Influenza like illness, secondary to SARS-CoV2 infection as detected by a reverse-transcription polymerase chain reaction (RT-PCR). Three weeks later, patient had recurrence of fever associated with throat pain, neck swelling, and a rash followed by shock and multiorgan failure. Laboratory work revealed elevated inflammatory markers. His throat swab for SARS-CoV-2 by RT-PCR was negative and SARS-CoV-2 IgG antibody was reactive.In view of presentation like multi-inflammatory syndrome in children (MIS-C) a probable diagnosis of MIS-A was made. He was successfully treated with intravenous Immunoglobulin and pulse dose Methylprednisolone. This case highlights the need to remain vigilant for multisystem inflammatory syndromes in the COVID-19 affected patients.[

Effectiveness of pulse dose methyl prednisolone in management of COVID 19: A systematic review and meta-analysis of observational studies.

Till date, only systemic corticosteroids have demonstrated definite mortality benefit in management of COVID 19 in various studies. Still certain questions regarding the appropriate dose, duration and timing of corticosteroids remain unanswered. For this reason, the study was planned to determine the efficacy and safety of the pulse dose methyl prednisolone in management of COVID 19 from the publicly available evidence. PubMed, the Cochrane library, ClinicalTrials.gov and medRxiv were searched for articles reporting the use of pulse dose methyl prednisolone in COVID 19 from inception till 31st May, 2021. Odds ratios (ORs) were calculated for estimation of pooled effect by using random effect model and heterogeneity was checked by using I2 statistics. Twelve studies (11 observational and 1 RCT) were included in the systematic review. A total of 3110 patients from 9 studies were included in the meta-analysis. Though the use of pulse dose methyl prednisolone demonstrated statistically significant mortality benefit in comparison to usual care (OR=0.71, 95% CI: 0.51 to 0.97, [P=0.03]), (I2= 21%) with calculated Number needed to treat (NNT) of 23.5, there was no statistically significant difference between the use of pulse dose and low dose corticosteroid (OR=0.66, 95% CI: 0.44 to 1.01, [(P=0.05]), (I2= 25%) and the NNT is 23.5. Incidence of adverse events were similar across all the groups. The grade of evidence for primary outcome was of moderate certainty. This meta-analysis concurs with the previous reports regarding the use of corticosteroid in COVID 19 in comparison to usual care. However, for both the primary and secondary outcome, the study did not find any statistically significant difference between the use of pulse dose methyl prednisolone and low dose corticosteroid to treat COVID 19 patients.

Longitudinally Extensive Transverse Myelitis: One Disease, Variable Outcomes-A Case Series.

Longitudinal extensive transverse myelitis (LETM) is a rare form of widespread inflammation of the spinal cord causing T2 hyperintensity in spinal magnetic resonance imaging (MRI) extending across three or more vertebral segments. It is an acute onset of sensory, motor, and autonomic dysfunction of variable etiology with a likely poor outcome. We present a case series of three cases wherein children between the ages of 4 and 13 years had diverse symptoms from gradual painless loss of vision in both eyes with headache, vomiting and seizure, and a normal central nervous system examination except involvement of the optic nerve to another child with abdominal pain, urinary retention and constipation for 3 days with exaggerated DTR, and patchy sensory loss without any definite sensory level, and to the third child with fever and weakness of lower limbs, hypotonia and grade 1–2/5 power in lower limbs and normal upper limb power. Contrast-enhanced MRI spine of all children showed long segment T2 hyperintensity with variable involvement of the brain. The first two children were treated with pulsed dose methylprednisolone, and the last child received intravenous immunoglobulin followed by methylprednisolone. All were followed with oral prednisolone. LETM has a varied presentation with different etiologies. Antineuromyelitis optica immunoglobulin G (IgG) antibody (Aquaporin-4 IgG) and antimyelin oligodendrocyte glycoprotein antibody are strongly recommended though they may not be locally available or not affordable. Early and aggressive immunomodulatory therapy may help faster recovery, as did with two of our three children.

Presence and Implications of Anti-Angiotensin Converting Enzyme-2 Immunoglobulin M Antibodies in Anti-Melanoma-Differentiation-Associated 5 Dermatomyositis.

ObjectivePatients with anti‐melanoma‐differentiation‐associated 5 (anti‐MDA5)‐positive dermatomyositis (DM) share several striking similarities to patients with SARS‐CoV‐2. Our objective was to assess the prevalence of anti‐angiotensin converting enzyme‐2 (ACE2) immunoglobulin M (IgM) antibodies, found in patients with severe SARS‐CoV‐2, in two independent anti‐MDA5‐positive DM cohorts.MethodsAnti‐ACE2 IgM antibodies were assayed by enzyme‐linked immunosorbent assay (ELISA) in two anti‐MDA5‐positive DM cohorts: a predominantly outpatient North American cohort (n = 52) and a Japanese cohort enriched for new‐onset disease (n = 32). Additionally, 118 patients with SARS‐CoV‐2 with a spectrum of clinical severity were tested for anti‐MDA5 antibodies by ELISA.ResultsFive of fifty‐two (9.6%) North American patients and five of thirty‐two (15%) Japanese patients were positive for anti‐ACE2 IgM. In the North American cohort, all five patients with anti‐ACE2 IgM antibodies had proximal muscle weakness, had interstitial lung disease, were significantly more likely to receive pulse dose methylprednisolone (80% vs 30%, P = 0.043), and had worse forced vital capacity (median 59% predicted vs 78%, P = 0.056) compared with the anti‐ACE2‐IgM‐negative group. In the Japanese cohort, all five anti‐ACE2‐IgM‐positive patients also required pulse dose methylprednisolone, and three of five (60%) patients died. Japanese patients with anti‐ACE2 IgM had significantly worse oxygenation, as defined by a lower partial pressure of oxygen (PaO2)/fraction of inspired oxygen (FiO2) ratio (233 vs 390, P = 0.021), and a higher alveolar‐arterial oxygenation gradient (91 vs 23 mm Hg, P = 0.024) than the IgM‐negative group.ConclusionWe describe anti‐ACE2 IgM autoantibodies in two independent cohorts with anti‐MDA5‐positive DM. These autoantibodies may be biomarkers for severe disease and provide insight into disease pathogenesis.

Lupus Eritematosus Sistemik pada Pria

Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by presence of nucleus autoantibody and affected multiple organ. Systemic lupus erythematosus is more common in women than men with ratio 2:1 to 15:1. Men with SLE often have a more aggressive clinical course, lead to a poorer prognosis compared with women with SLE. Case Report: A man, 29 years old came to hospital with main complain joint pain increased since 1 week ago, accompanied with red spot on face, trunk, hands, foot, and back, hair loss, swollen leg, mouth ulcer, and fatique. Malar rash and discoid rash were identified from physical examination. From laboratorium, ANA profile was positive for RNP/Sm, Sm, dsDNA, and histone. Skin biopsy showed a lupus discoid. Conclusion: The patient was treated with pulse-dose methylprednisolone for 3 days and showed a good response clinically.

Akut Dissemine Ensefalomiyelite Bağlı Periferik Fasiyal Sinir Felci Gelişen Bir Çocuk Hasta

Akut dissemine ensefalomiyelit (ADEM), nörogörüntülemede multifokal demiyelinasyon lezyonlarının kanıtıyla birlikte yaygın nörolojik belirti ve semptomlarla karakterize, santral sinir sisteminin monofazik akut demiyelinizan bir bozukluğu olarak kabul edilir. Yaygın görülen semptomlar letarji, ateş, kusma, baş ağrısı, meningeal belirtiler ve nöbetlerdir. Nörolojik belirtiler arasında duyusal değişiklikler, çoklu kraniyal sinir tutulumu, kuadripleji ve parapleji, distoni ve koreiform hareketler, ataksi, nistagmus, mesane tutulumu, konuşma bozukluğu ve çift görme vardır. Periferik fasiyal sinir felci nadiren eşlik eder. Akut dissemine ensefalomiyelit ile sağ periferik fasiyal sinir felci gelişen 7 yaşındaki bir kız çocuğu olgusunu sunuyoruz. Hastaneye görme azlığı, depresif ruh hali, yürüme bozukluğu ve yüz ifadelerinde bozulma yakınmaları ile başvurdu. Horizontal ve vertikal nistagmus, sağ periferik yüz felci, ataksik yürüyüş ve derin tendon reflekslerinde artış vardı. Beyin omurilik sıvısı (BOS) incelemesi normaldi. Serebral manyetik rezonans görüntülemede (MRG) çoklu beyaz cevher lezyonları görüldü. Yüksek doz metilprednizolon pulse tedavisi ile klinik bulgularda belirgin bir iyileşme gözlemledik.

Optic neuritis in a teenage girl with granulomatosis with polyangiitis

Introduction. Granulomatosis with polyangiitis (GPA), formerly known as Wegener?s granulomatosis, is characterized by necrotizing granulomatous inflammation in various tissues, including blood vessels, but primarily in the respiratory tract and kidneys. Clinical manifestations can be diverse, including inflammation of the eye and adnexa. Optic neuritis is a very rare ophthalmological manifestation of GPA, not previously described in a teenager. Case report. We presented a case of a 16-year-old girl with a rare extrapulmonary manifestation of GPA. The girl had a previous history of GPA and complained of a sudden blurred vision in the left eye. She was promptly referred to an ophthalmologist who noted a decreased visual acuity of 20/400 in the left eye. Colour vision was impaired in the spectrum of red colour. Clinical examination revealed normal anterior segment findings. On ophthalmoscopy, the left optic nerve oedema was noted. Urgent computed tomography of the left orbit showed a soft tissue mass around the optic nerve in the apex of the orbit. Magnetic resonance imaging confirmed the diagnosis of optic perineuritis. After pulse doses of methylprednisolone, the girl achieved complete resolution of vision in the left eye. Conclusion. If untreated, inflammation of the optic nerve can lead to a permanent loss of vision. Prompt diagnostic and adequate treatment of patients with GPA is needed in order to prevent vision-threatening complications and control the systemic disease.

SUCCESSFUL TREATMENT WITH PLASMAPHERESIS AND IMMUNOSUPPRESSANTS IN ANCA-NEGATIVE GOOD PASTURE SYNDROME: A RARE CASE REPORT

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Goodpasture syndrome (GPS), or Anti-glomerular basement membrane disease is a rare autoimmune disease with an annual incidence of 0.5-1.6 cases per million [1]. We report a case of cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) negative GPS disease where combined immunosuppressant and plasmapheresis treatment resulted in significant improvement of both hemoptysis and renal function. CASE PRESENTATION: A 19-year-old female with past medical history of obesity, asthma and chronic cough presented with shortness of breath, and hemoptysis of two week duration despite being treated with several courses of antibiotics for presumed recurrent pneumonia. She was found to be hypertensive on arrival (BP 163/95 mmHg). Work up revealed elevated creatinine, significant hematuria and proteinuria. CT chest demonstrated bilateral multifocal patchy infiltrates suspicious for pneumo-renal syndrome. She was empirically started on pulse dose methylprednisolone. Autoimmune workup revealed positive anti-glomerular basement membrane (anti-GBM) antibodies (36U ; normal value <20U) and negative C-ANCA. Renal biopsy showed acute necrotizing fibrocellular crescents involving more than two third of the glomeruli and linear GBM staining for IgG suggestive of anti-GBM nephritis confirming the diagnosis of GPS. Treatment with five rounds of plasmapheresis and immunosuppressive therapy with cyclophosphamide followed by extended release prednisone taper resulted in significant clinical improvement. DISCUSSION: GPS is characterized by auto reactivity to antigens in type IV collagen chains present in the glomeruli and alveolar basement membrane capillaries resulting in crescentic glomerulonephritis and diffuse pulmonary alveolar hemorrhage[2]. Additionally, ANCA is associated with GBM in 20-60% of the cases. The current treatment includes plasma exchange(PE) which involves removal of circulating anti-GBM antibodies and other mediators of inflammation, and immunosuppressants that minimize new antibody formation[3]. Although PE is effective in the treatment of GPS, the efficacy of combined immunosuppressant with PE is not well established. Our case is unique that the combined treatment with immunosuppressants and plasmapheresis resulted in a significant improvement of both hemoptysis and renal function in a ANCA negative good pasture’s disease. As GPS is associated with irreversible renal damage and high mortality rate, early and appropriate treatment may reverse the kidney damage and prevent the need for hemodialysis[2]. However, further multi-center clinical trials are required to establish its efficacy in the treatment of GBM. CONCLUSIONS: Our case highlights that PE in combination with cyclophosphamide resulted in significant improvement when compared to treatment with PE alone as reported in the literature. Early diagnosis and treatment can result in favorable outcomes in patients with GPS. Reference #1: Marques, C., et al., Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients. Front Immunol, 2019. 10: p. 1665. Reference #2: Apaydin, S., The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis. Transfus Apher Sci, 2018. 57(1): p. 8-12. Reference #3: Prendecki, M. and C. Pusey, Plasma exchange in anti-glomerular basement membrane disease. Presse Med, 2019. 48(11 Pt 2): p. 328-337. DISCLOSURES: No relevant relationships by Ioana Amzuta, source=Web Response No relevant relationships by Anupama B K, source=Web Response No relevant relationships by Japjot Chahal, source=Web Response No relevant relationships by Christopher Ramos, source=Web Response No relevant relationships by Akhila sunkara, source=Web Response

IMMUNE CHECK POINT INHIBITOR-INDUCED ORGANIZING PNEUMONIA IN A LUNG TRANSPLANT PATIENT

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Immune check point inhibitors (ICI) are an effective therapy in treatment of multiple malignancies. Ipilimumab, a CTLA-4 antibody was the first ICI to be approved for advanced melanoma, followed by pembrolizumab, a PD1 inhibitor(1)(2). A myriad of immune related adverse effects have been observed with use of checkpoint inhibitors, due to T cell activation. ICI up regulate immune response to cancer but also leads to rejection of transplanted organs. Here we present a case of ICI pneumonitis after treatment with pembrolizumab in a lung transplant patient. CASE PRESENTATION: An 80-year-old Caucasian male received bilateral lung allografts in 2003 for idiopathic pulmonary fibrosis. In 2014, he was diagnosed with malignant melanoma and squamous cell carcinoma of scalp and neck, and underwent wide local excision with radiation therapy. He received two cycles of pembrolizumab for recurrence of melanoma (BRAF wildtype) after which he developed acute hypoxemic respiratory failure requiring mechanical ventilation. The patient received broad spectrum antibiotics and pulse dose methyl prednisolone for suspected transplant rejection. Bronchoalveolar lavage showed bilateral alveolar hemorrhage and trans-bronchial biopsies of the left lower lobe and lingula showed acute organizing pneumonia. With the concern for ICI pneumonitis versus ICI induced rejection of lung allograft the patient was treated with pulse dose steroids and then a prolonged steroid taper. DISCUSSION: Multiple adverse events have been reported with ICI including pulmonary toxicity. In a meta-analysis of 6360 patients, the incidence of pneumonitis from anti-PD-1 immunotherapy was 2.92% (3). Increased T-cell activity, inflammatory cytokines, and enhanced complement-mediated inflammation is suggested as the mechanism. ICI pneumonitis may manifest as cryptogenic organizing pneumonia, interstitial pneumonia, hypersensitivity pneumonitis, or acute respiratory distress syndrome patterns (4). Lung toxicities are graded dependent on severity of symptoms and extent of radiological involvement and American Society of Clinical Oncology provides expert consensus on treatment recommendations. Current guidelines indicated the discontinuation of ICI and initiation of corticosteroids in our patient with grade 3 toxicity. In refractory cases, anti-TNF-a agent infliximab, has been used. Allograft rejection must also be considered in lung transplant patients who undergo ICI therapy. Infectious pneumonia must also be excluded as this can be fatal if left untreated (5). CONCLUSIONS: Our patient presented with ICI related organizing pneumonia in transplanted lungs, which has been reported in few case reports with favorable response to high dose steroids. Awareness of potential complications of ICI especially in transplant population facilitates early recognition and prompt treatment with improved outcomes. Reference #1: 1) Alexander W. The Checkpoint Immunotherapy Revolution: What Started as a Trickle Has Become a Flood, Despite Some Daunting Adverse Effects; New Drugs, Indications, and Combinations Continue to Emerge. P T. 2016;41(3):185–191. Reference #2: 2) Robert, C., Schachter, J., Long, G. V., Arance, A., Grob, J. J., Mortier, L., … Ribas, A. (2015). Pembrolizumab versus Ipilimumab in Advanced Melanoma. New England Journal of Medicine, 372(26), 2521–2532. doi: 10.1056/nejmoa1503093 Reference #3: 3) Nishino M, Giobbie-Hurder A, Hatabu H, Ramaiya NH, Hodi FS. Incidence of Programmed Cell Death 1 Inhibitor–Related Pneumonitis in Patients With Advanced Cancer: A Systematic Review and Meta-analysis. JAMA Oncol. 2016;2(12):1607–1616. doi:10.1001/jamaoncol.2016.2453 4) Nishino, M., Giobbie-Hurder, A., Hatabu, H., Ramaiya, N. H., & Hodi, F. S. (2016). Incidence of Programmed Cell Death 1 Inhibitor–Related Pneumonitis in Patients With Advanced Cancer. JAMA Oncology, 2(12), 1607. doi: 10.1001/jamaoncol.2016.2453 5) Jun, J., Lee, S. R., Lee, J. Y., Choi, M. J., Noh, J. Y., Cheong, H. J., … Song, J. Y. (2019). Pneumonitis and concomitant bacterial pneumonia in patients receiving pembrolizumab treatment: Three case reports and literature review. Medicine, 98(25), e16158. doi:10.1097/MD.000000000001615 DISCLOSURES: no disclosure on file for Deepa Gotur; No relevant relationships by Uchenne Mbaraonye, source=Web Response no disclosure on file for Cindy Nguyen; no disclosure on file for Lily Romero Karam; Speaker/Speaker's Bureau relationship with Astellas Pharma US, Inc. Please note: $1001 - $5000 Added 03/10/2020 by Simon Yau, source=Web Response, value=Salary